| glucose-6-phosphatase | <enzyme> An enzyme that catalyses the conversion of d-glucose-6-phosphate and water to d-glucose and orthophosphate. This enzyme is deficient in glycogen storage disease Ia. Chemical name: D-Glucose-6-phosphate phosphohydrolase Registry number: EC 3.1.3.9 (12 Dec 1998) |
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| glucose-6-phosphatase hepatorenal glycogenosis | Glycogenosis due to glucose-6-phosphatase deficiency, resulting in accumulation of excessive amounts of glycogen of normal chemical structure, particularly in liver and kidney. Synonym: Gierke's disease, glucose-6-phosphatase hepatorenal glycogenosis, von Gierke's disease. (05 Mar 2000) |
Synonyms : Glucose 6-Phosphatase, Glucose-6-Phosphate Phosphohydrolase, Glucose 6 Phosphatase
| glucose-6-phosphatase |
[EC 3.1.3.9] an enzyme of the hydrolase class that catalyzes the dephosphorylation of glucose 6-phosphate. It occurs in the endoplasmic reticulum of liver, kidney, and intestinal mucosa, but not of muscle, and its reaction is the principal route for hepatic gluconeogenesis, controlling blood glucose concentrations. Deficiency of the enzyme, an autosomal recessive trait, results in glycogen storage disease, type I.
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